کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1905113 1534690 2011 9 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Myelin genes are downregulated in canine fucosidosis
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی سالمندی
پیش نمایش صفحه اول مقاله
Myelin genes are downregulated in canine fucosidosis
چکیده انگلیسی

The processes regulating the complex neurodegenerative cascade of vacuolation, neuroinflammation, neuronal loss and myelin deficits in fucosidosis, a neurological lysosomal storage disorder, remain unclear. To elucidate these processes the gene expression profile of the cerebral cortex from untreated and intrathecal enzyme replacement therapy treated fucosidosis pups and age-matched unaffected controls were examined. Neuroinflammation and cell death processes were identified to have a major role in fucosidosis pathophysiology with 37% of differentially expressed (DE) genes involved in these processes. Critical, specific, early decreases in expression levels of key genes in myelin assembly were identified by gene expression profiling, including myelin-associated glycoprotein (MAG), myelin and lymphocyte protein (MAL), and oligodendrocyte myelin paranodal and inner loop protein (OPALIN). These gene expression changes may be indicative of early neuronal loss causing reduced electrical impulses required for oligodendrocyte maturation.

Research highlights
► Oligodendrocyte and myelin genes are downregulated in early canine fucosidosis.
► Neuroinflammation and apoptosis are key disease processes.
► Low dose, monthly enzyme replacement therapy does not significantly affect global gene expression.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1812, Issue 11, November 2011, Pages 1418–1426
نویسندگان
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